Pediatric investigation publishes China’s first clinical guidelines for juvenile-onset recurrent respiratory papillomatosis

Juvenile-onset recurrent respiratory papillomatosis (JORRP) is a rare disease of the respiratory tissues in children caused by low-risk human papillomavirus (HPV) types 6 and 11, leading to recurrent wart-like growths called papillomas on the respiratory tract. It often appears in children as young as two or three years, resulting in chronic hoarseness, breathing difficulties, and repeated airway obstruction. Many patients endure multiple surgeries—some over 40 in their lifetime—to maintain airway function and prevent life-threatening complications.

In China, the burden of JORRP is exacerbated by the absence of national epidemiological data, making it difficult to understand the true scope of the disease or allocate resources effectively. Clinical practices vary widely due to a lack of standardized, evidence-based guidelines tailored to the local healthcare system. As a result, patients with JORRP and their families experience delayed diagnoses, fragmented care, and limited access to appropriate treatment.

While international protocols—such as those issued by the International Pediatric Otolaryngology Group (IPOG) in 2020—provide comprehensive recommendations for managing JORRP, they are primarily designed for healthcare in North America and Europe. As a result, their direct implementation poses logistical and clinical challenges in the Chinese context. To bridge this gap, a multidisciplinary group of Chinese experts developed context-specific clinical guidelines for standardizing diagnosis and treatment practices nationwide, ensuring more equitable and effective care for children with JORRP. The guidelines for diagnosing and treating JORRP were published in Pediatric Investigation on 17 March 2025.

“For the first time, we’ve created a national roadmap for managing this complex and rare disease,” says Dr. Xi Ni, corresponding author and a senior physician at the Department of Otorhinolaryngology, Beijing Children’s Hospital. He adds, “The working group, made up of multidisciplinary experts with extensive frontline experience, undertook rigorous research—ranging from Delphi consensus rounds to systematic reviews and meta-analyses. Our goal was to distill this evidence into 24 clear, actionable recommendations that address the unique clinical realities of the Chinese healthcare system.”

Accordingly, the group emphasizes early recognition as critical in managing JORRP. They urge clinicians to treat persistent hoarseness lasting over two months, inspiratory wheezing (a high-pitched, whistling sound heard during inhalation that may indicate airway narrowing), and cyanosis (a bluish tint to the skin or lips caused by low oxygen in the blood) as urgent warning signs. Once the child’s vital signs are stable, they recommend performing flexible laryngopharyngoscopy—a minimally invasive procedure using a thin, bendable camera inserted through the nose to visualize the voice box and upper airway.

The group strongly supports using the Derkay scoring system, which evaluates the number and anatomical location of papillomas and the severity of symptoms, to assess the extensiveness of JORRP in affected patients. They stress the need to distinguish between invasive and non-invasive forms of JORRP. They also identified high-risk populations requiring closer and longer follow-up: children with early disease onset (typically before age four), those infected with HPV type 11 (a more aggressive subtype), those whose mothers had condyloma acuminatum (genital warts) during pregnancy, and children from low-income families who may have limited access to timely care.

Also, the group recommends against delays in surgical intervention, particularly when the child’s trachea is affected. Surgeons should prioritize preserving vocal ligaments and minimizing scarring. Tracheostomy (a technique involving surgical opening in the windpipe) should be considered only when strictly necessary and reversed at the earliest opportunity. The American Society of Anesthesiologists physical status score should guide airway evaluation before surgery. The group noted that endotracheal intubation (inserting a tube into the windpipe) for anesthesia. However, anesthesiologists may also consider jet ventilation (a method of delivering oxygen using rapid bursts).

In addition, the group emphasized individualization of follow-up care based on patient age, HPV subtype, and surgical history. They recommend that doctors should advise families to seek immediate care if symptoms return to affected children. They also cautiously support using bevacizumab (a drug that inhibits abnormal blood vessel formation in papillomas) for treating fast progressing or extensive JORRP cases.

“We hope this guideline will empower clinicians across China to diagnose JORRP earlier, intervene more precisely, and ultimately improve long-term outcomes for children living with this condition,” remarks Dr. Ni. Indeed, these evidence-based guidelines mark a major step forward in standardizing care and could significantly improve the quality of life for pediatric patients affected by this challenging disease.

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Reference

Authors’ Affiliated Organizations: Working Group of Chinese Guideline for the Diagnosis and Treatment of JORRP, Subspecialty Group of Pediatrics, Society of Otorhinolaryngology Head and Neck Surgery, Chinese Medical Association, and Editorial Board of Chinese Journal of Otorhinolaryngology Head and Neck Surgery

DOI: https://doi.org/10.1002/ped4.12464